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Pulmonary alveolar proteinosis
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Pulmonary alveolar proteinosis

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Contributors: Casey Silver MD, Mary Anne Morgan MD, Michael W. Winter MD
Other Resources UpToDate PubMed

Synopsis

Pulmonary alveolar proteinosis (PAP) is a rare lung disorder characterized by the filling of the pulmonary alveoli with proteinaceous material. This material consists of surfactant phospholipids and apoproteins and stains positive with periodic acid-Schiff (PAS) stain. Alveolar architecture is typically preserved, and inflammatory cell infiltration is minimal.

There are 3 categories of the condition:
  1. Autoimmune and hereditary PAP – Causes disruption of granulocyte-macrophage colony-stimulating factor (GM-CSF).
  2. Congenital PAP – Genetic disorder of surfactant production; presents in neonates.
  3. Secondary PAP – Associated with mineral dust exposure, hematologic malignancies, and allogenic hematopoietic cell transplantation.
Typical age of presentation in adults with PAP is between 40 and 50 years. Smoking is a significant risk factor. Some patients will have other autoimmune diseases.

Signs and symptoms include dyspnea, particularly on exertion, cough, and low-grade fever. The classic radiographic pattern is "crazy paving" on high-resolution CT, but other patterns may be observed.

Neonates with congenital PAP present in the first 1-2 years of life with symptoms ranging from severe respiratory failure in the neonatal period to insidious chronic interstitial lung disease in later months.

Codes

ICD10CM:
J84.01 – Alveolar proteinosis

SNOMEDCT:
10501004 – Pulmonary alveolar proteinosis

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 07/12/2019
Last Updated: 07/17/2019
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Pulmonary alveolar proteinosis
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Pulmonary alveolar proteinosis : Exertional dyspnea, Malaise, Pleuritic chest pain, Weight loss, Low grade fever, Dry cough
Copyright © 2019 VisualDx®. All rights reserved.